Embryonal carcinoma of the ovary is an extremely rare tumor. It can be distinguished from a choriocarcinoma by the absence of syncytiotrophoblastic and cytotrophoblastic cells. Embryonal carcinomas may be hormonally active, with some patients developing precocious pseudopuberty or irregular bleeding. The presentation is similar to that of the EST. The patient’s ages ranged between 4 and 28 years (median was 14 years) in one series. The primary lesions tend to be larger than 15 cm in diameter, and about two thirds are confined to one ovary at the time of presentation. As with the pure EST, these lesions often secrete AFP and hCG, and these markers are useful for following the response to subsequent therapy. The treatment of embryonal carcinomas is the same as for the EST, that is, the performance of a unilateral oophorectomy or salpingo-oophorectomy followed by combination chemotherapy with BEP.